Supernumerary Teeth: Case Comparison and Review ...

Supernumerary Teeth: Case Comparison and Review of the Literature By Richard Vargo, DMD, Elizabeth Ann Bilodeau, DMD, MD, MSEd, and Anitha Potluri, BDS, DMD, MDS yperdontia is when the number of teeth exceeds the number found in a normal dentition, and these extra teeth are referred to as supernumerary teeth.1 Generally, supernumeraries are more common in men, more common in the maxilla, and more prevalent in the permanent dentition. 1 Complications associated with supernumerary teeth include dental impaction, delayed eruption, ectopic eruption, crowding, spacing, and the formation of dentigerous cysts. 1 Most frequently, patients present with a single supernumerary tooth - with up to 92.5 percent of cases involving a single supernumerary tooth. 2 In fact, the presentation of more than two supernumerary teeth is rare and represents less than one percent of all cases. 1 The most common supernumerary tooth location is between the maxillary central incisors, referred to as a me-

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siodens (Figure lA, B, C).3 Supplemental supernumeraries are duplicate permanent teeth.3 The most common supplemental teeth are lateral incisors, followed by premolars, and then the molars. 3 Supernumerary teeth associated with molars are called distomolars or paramolars. Supernumerary molars distal to the molars are distomolars (Figure 2). 3 Those that are buccal or lingual can be referred to as paramolars. 3 Numerous syndromes have associations with supernumerary teeth. We report two cases of rare autosomal dominant syndromes associated with multiple supernumerary teeth. The clinical and radiographic features of each case are discussed.

Case 1 A 15-year-old female presented with her parents for

JOURNAL OF THE MICHIGAN DENTAL ASSOCIATION • DECEMBER 2015

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evaluation. Her mother reported that no one to date could figure out what was wrong with her daughter's teeth. She noted that her daughter still has most of her "baby" teeth and only a few of her permanent teeth. She had been referred to the clinic by an outside orthodontist. The patient's medical history was significant for hearing problems and antibiotic allergies. She presented with short stature (with a height of 4 feet 8 inches). A panoramic radio-

graph revealed the presence of numerous impacted supernumerary teeth, carious retained primary teeth, and unerupted permanent teeth (Figure 3). The patient also demonstrated that she was able to closely approximate her shoulders (Figure 4, Page 32). Subtle hypertelorism, frontal bossing, and a fiat nasal bridge were noted. The presence of underdeveloped clavicles, multiple supernumerary teeth, and failure of eruption of several permanent teeth was suggestive of cleidocranial dysplasia. Upon explaining the syndrome to the parents, they mentioned that the patient also had a "soft forehead" at birth. With a working diagnosis of cleidocranial dysplasia, the patient and her parents opted to forego orthodontic therapy in favor of extracting the primary teeth and unerupted permanent teeth followed by the placement of dental implants and overdenture fabrication.

Case 2 A 19-year-old female presented with pain in her left posterior mandible and right mandible. The patient's medical history was significant for Gardner syndrome, and an outside pediatrician was monitoring her. She stated that her sister also

has Gardner syndrome and that her father died as a result of it. About a year before the appointment, the patient stated that her colonoscopy revealed cancerous polyps. She subsequently underwent a subtotal colectomy with ileorectal anastomosis. She stated that she has vitamin deficiencies and electrolyte imbalances resulting from her surgery but was unable to elaborate further. She takes methadone for chronic abdominal pain from her surgery. Intraoral examination revealed no evident clinical caries in the areas of interest. A panoramic radiograph revealed the presence of impacted teeth, supernumerary teeth, odontomas, and congenitally missing teeth (Figure 5, Page 32). Multiple supernumerary teeth or odontomas were noted in the following tooth-bearing areas: #19/20, #4/5, #6/7 and #10/11. Teeth #17 and #32 were congenitally missing. Overall, the mandibular bone had a highly dense, diffuse radiopaque appearance. Examination of her skin revealed no epidermoid cysts, and she stated that she had never had any skin lesions. Clinical examination and testing did not reveal any periapical or pulpal etiology of the pain. It was determined that her pain was intraosseous

Figure 3: Panoramic radiograph showing retained carious primary teeth, impacted permanent teeth, and multiple supernumerary teeth.


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Supernumerary Teeth

(Continued from Page 31) in nature, most likely from the radiodense areas, which are consistent with osteomas. The patient was referred to an oral and maxillofacial surgeon for further evaluation.

Discussion The etiology of hyperdontia is not well-understood. The cause can be multifactorial (both environmental and genetic).3 Multiple supernumerary teeth are associated with syndromes like Cleidocranial dysplasia, Gardner, Ehlers-Danlos, Ellis-van Creveld, Sturge-Weber, Apert, Crouzon and many more. 3 The presence of supernumerary teeth may not cause any complications; however, in some cases, they may cause delayed erup-

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tion, tooth displacement, crowding, root resorption and loss of vitality of adjacent teeth. 3 Early recognition of their presence or any conditions associated with them will be helpful for early intervention and correction. Cleidocranial dysplasia, also known as cleidocranial dysostosis, is an autosomal dominant skeletal dysplasia syndrome that affects both bone and teeth.4·5·6 It is caused by mutations of the transcription factor RUNX2. 4·5·6 Located on chromosome 6p21, this gene is considered a master gene in the formation of bone and dental tissues.4·5·6 Cleidocranial dysplasia has a high penetrance and a wide variation in the clinical expressivity.6·7 Since its first description in 1898 by Pierre Marie and Paul Sainton, over 1,000 cases have been published in the medicalliterature.5 Most of these are presentations of single cases due to the rareness of the disorder, which has an incidence of 1:1,000,000.5 Cleidocranial dysplasia primarily affects bones that undergo intramembranous ossification-that is, the calvaria and also the lateral end of the clavicular bones. 5 The main symptoms of cleidocranial dysplasia are pathognomonic deformity of the skull resulting in persistently open skull sutures with bulging calvaria, aplasia or hypoplasia of one or both the clavicles, delayed ossification of the

fontanelles , frontal and parietal bone bossing, wide pubic symphysis, short middle phalanges of the fifth fingers, supernumerary teeth, poor development of the premaxilla, and short stature.4·7 The skull base is dysplastic and reduced in growth.5 Patients often present with an increased skull width, resulting in hypertelorism and brachycephaly.4·5 Cleidocranial dysplasia can have forty-eight distinct phenotypic features, including hearing loss. 4 The patient in this case had impaired hearing, which is likely related to her syndrome. Characteristically, the clavicles of patients with cleidocranial dysplasia can be approximated in the midline.4·5 Skeletal symptom11 such as this clavicular sign and a hypoplastic maxilla are exhibited in over 84.3 percent ofpatients. 5 As stated, these patients often have a midface deficiency caused by maxillary hypoplasia.4·5 Since only the permanent dentition is affected in cleidocranial dysplasia, the midface deficiency can be masked before the pubertal growth spurt. 5 Young cleidocranial dysplasia patients have relatively normal jaw proportions and morphology of the mandible, while older patients tend to express the typical signs of cleidocranial dysplasia. 8 This occurs because of the pronounced horizontal mandibular growth resulting from a lack of verti-


cal maxillary growth as well as impaired eruption of the permanent teeth. All craniofacial regions are affected with cleidocranial dysplasia to a greater or lesser extent, and this abnormal craniofacial morphology is explained by the skeletal dysplasia.5 Chief intraoral findings include retained deciduous dentition, delayed eruption or retention of the permanent dentition, multiple supernumerary teeth, and a high palate. 5 •7 Specifically, supernumerary teeth and eruption failure are expressed in over 93.5 percent of cleidocranial dysplasia patients. 5 In one report, the number of supernumerary teeth ranged from 2-20 teeth, and the number of impacted teeth ranged from 1-20 teeth. 7 Dental alterations in cleidocranial dysplasia often result in severe malocclusion, requiring a long and complex dental treatment plan.7 Cleidocranial dysplasia should be suspected in patients with short stature and abnormalities of their clavicles, calvarium, and teeth.4 The diagnosis oftypical cleidocranial dysplasia is generally not challenging for the clinician since it can be differentiated easily from rickets, dwarfism, and osteogenesis imperfecta. 4 However, the diagnosis can be difficult if typical features are not clearly expressed. 4 •5 Timely recognition and genetic counseling is required because of the possibility of passing on the affected gene unknowingly. 4 Moreover, early diagnosis of cleidocranial dysplasia allows practitioners to initiate treatment sooner to hopefully minimize the extent of lateral surgical and orthodontic intervention.5 Gardner syndrome is a rare, autosomal dominant syndrome that was first described in 1953.8 It is characterized by multiple colorectal adenomatous polyps and extraintestinal findings including osteomas, epidermoid cysts, dental abnormalities, other cutaneous lesions, and desmoid tumors. 8 · 11 The prevalence of Gardner syndrome ranges between 1 in 8,000 and 1 in 12,000 individuals. 8 •11 Thirty percent of cases result from a new dominant mutation.10·11 Gardner syndrome is caused by a mutation in the adenomatous polyposis coli gene

located at chromosome 5q21. 8 · 11 This same gene is mutated in familial adenomatous polyposis (FAP), and researchers consider Gardner syndrome to be a clinical variation of FAP.9 Therefore, together with FAP, attenuated FAP, and Turcot syndrome, Gardner syndrome comprises a group of syndromes known as the APC-associated polyposis conditions.10 Inactivation of the APC gene leads to accumulation of the protooncogene beta-catenin, dysregulation of the cell cycle, and chromosomal instabilityP The site of the mutation of the APC gene determines the manifestations of Gardner syndrome and specific codon mutations are related to the specific extraintestinal manifestations .10 Specifically, the most frequent APC mutation is located at codon 1309, and mutations here lead to the development of a high number of colonic adenomas at an early age. 10 More distal APC mutations in the codons 1395-1493 are associated with higher rates of epidermoid cysts, osteomas, and desmoid tumors. 10 The gastrointestinal manifestations of Gardner syndrome include colonic adenomatous polyps, gastric and small intestinal adenomatous polyps, and periampullary carcinomas.10 Colorectal polyps become visi-

ble at puberty and may transform into carcinoma by the fourth decade of life.8 •10·11 •13 However, malignant change has been reported in patients as early as twenty years of age. 9 Gardner syndrome intestinal polyps have a 100% risk of undergoing malignant transformation to colorectal adenocarcinoma; consequently, early identification and surgical inter vention are important to prolong the life of the patient because the polyposis may be fatal if not treated.8 •9•11 The presenting symptoms are anemia, rectal bleeding, constipation, cramping and abdominal pain, diarrhea, bowel obstruction, and mucous discharge. 10 Colonoscopy reveals the rectum and colon to be covered with polyps. 10 Colectomy to remove the gastrointestinal manifestations is advised at diagnosis considering the rapid malignant transformation potential, especially if more than 20 or 30 adenomas or multiple adenomas with advanced histology are detected. 10·13 Given the usual temporal presentation of the polyps, prophylactic resection should be performed before the twenty-fifth year of life.10 Duodenal cancer occurs in about 5 percent ofFAP patients following colectomy. 11 Because the oral and maxillofacial signs of Gardner syndrome usually

About the Authors Dr. Anitha Potluri is an associate professor of oral and maxillofacial radiology at the University of Pittsburgh School of Dental Medicine. She completed her residency and

ma~ter's in

dental sciences at the University of Connecticut . She completed her dental education at the University of Pittsburgh and HKES College of Gulbarga University in India.

Dr. Elizabeth Bilodeau is an associate professor of oral and maxillofacial pathology at the University of Pittsburgh School of Dental Medicine. She completed her residency and medical degree at the University of Pittsburgh. She completed her dental degree and master's in higher education administration at the University of Pennsylvania . Dr. Bilodeau can be contacted at [email protected].

Dr. Richard Vargo is a first year oral and maxillofacial pathology resident at the University of Pittsburgh School of Dental Medicine. He completed his dental degree at the University of Pittsburgh .


Dr. Bilodeau

Dr. Vargo

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Supernumerary Teeth

(Continued from Page 33) precede the gastrointestinal symptoms by years, the dentist may play an important role in the recognition and diagnosis of the syndrome. 9•10 A panoramic radiograph is a very effective means of detecting the multiple osteomas of the jaws and dental abnormalities that are characteristic of the syndrome. 8 Forty-six to 93 percent of patients with Gardner syndrome present with osteomatous lesions, and the average number of bone lesions ranges from 2.9-4.7_13 The osteomas are characterized by slow, continuous growth, and occur most frequently in the mandible, the outer cortex of the skull, and the paranasal sinuses. 8 The angle of the mandible is a particularly diagnostic site. 8 The osteomas may be either exostoses, often referred to as peripheral osteomas, or endostoses, which are detectable only radiographically. 8 If more than three osteomas are found, it is highly suggestive of Gardner syndrome. 8Another type of lesion has been described, which appears as a large and diffuse radiopaque cotton-wool like area in either jaw and is referred to as a widespread radiopaque lesion. 8 This can be confused with cement-osseous lesions such as florid cement-osseous dysplasia or familial gigantiform cementoma. 9 Pain is rarely observed in these patients, and the bone lesions are generally asymptomatic.8·10 Interestingly, the patient in this case did seem to have pain associated with her osteomatous lesions, as no odontogenic source could be determined. Dental abnormalities include impacted teeth, multiple supernumerary teeth, odontomas, and congenitally missing teeth. 8·9 •10·14 Dental abnormalities are present in around 30% of patients with Gardner syndrome. 8 Supernumerary teeth and impacted teeth are more frequent in patients with Gardner syndrome than in the normal population. 10 The highest incidence of dental abnormalities is found in patients with multiple os34

teomas. 8 Extractions in patients with this syndrome may be of increased difficulty due to the extremely dense nature of the alveolar bone. 10 The most frequent skin manifestations of Gardner syndrome are asymptomatic epidermoid cysts. 10 Multiple epidermoid cysts are present in around 50-65 percent of patients with Gardner syndrome. 8 The cysts arise prior to puberty and occur primarily on the face, scalp, and extremities ~ 8 · 10 Other skin manifestations include fibromas, neurofibromas, leiomyomas, and lipomas. 10 Desmoid tumors appear in 3.5-5.7 percent of patients. Desmoid tumors, which are slow growing deep fibromatoses that are histologically benign and have no metastatic potential, may occur in the skin of anterior abdominal wall or intra-abdominally. 8 The clinical diagnosis of Gardner syndrome is often difficult for clinicians to make because there is a great variation in the dental, osseous, and cutaneous findings. 9•10 While the patient in this case presented with a family history and an already diagnosed case of Gardner syndrome, her clinical findings provide a good example of this point. The patient only had a few supernumerary teeth and odontomas, and her bony lesions could easily be confused with florid cementa-osseous dysplasia or familial gigantiform cementoma, given the diffuse involvement of the mandible. Moreover, she had no characteristic cutaneous lesions. A dentist treating an undiagnosed patient with her presentation may not consider this syndrome, delaying potentially life-saving colonoscopy and subsequent treatment. Regardless of the diverse presentation of Gardner syndrome, timely diagnosis has implications for other family members. 8·11 The advantage of identifying the mutation in a family is that it allows gene carriers within the family to be accurately identified. 11 Conclusion

We report two rare cases of autosomal dominant syndromes that can be associated with multiple supernumerary teeth. As the general dentist

may be the initial person to screen these patients, the dentist should consider these syndromes if they see supernumerary teeth with delayed eruption of permanent teeth and then look closer for the other characteristic findings. Ultimately, prompt referral can help minimize the inevitable dental problems in the case of cleidocranial dysplasia and be potentially life-saving, in the case of Gardner syndrome.~

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This paper was peer-reviewed.
